Source:http://linkedlifedata.com/resource/pubmed/id/15572594
Switch to
| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
6
|
| pubmed:dateCreated |
2005-3-4
|
| pubmed:abstractText |
The binding of frizzled (Fzd) receptors by their Wnt ligands results in the inhibition of beta-catenin degradation and subsequent transcription of beta-catenin/LEF-inducible genes. The beta-catenin pathway is known to be involved in development, tumorigenesis, and stem cell self-renewal. In humans, the FZD9 gene lies in the region of chromosome 7q11.23 deleted in the neurodevelopmental disorder, Williams-Beuren syndrome (WBS). Fzd9-/- mice show no obvious features of WBS, but reveal a role for Fzd9 in lymphoid development and maturation. Fzd9-/- mice show pronounced splenomegaly, thymic atrophy, and lymphadenopathy with age, with accumulation of plasma cells in lymph nodes. There is a depletion of developing B cells in the bone marrow (BM), particularly in the pre-B stage where immunoglobulin heavy chains are expressed and the cells are undergoing clonal expansion prior to light chain rearrangement. The pre-B defect is partially intrinsic to the hematopoietic system; as in competitive BM reconstitution studies, Fzd9-/- -derived BM exhibits defective B-cell development when implanted into a wild-type host. Mature B cells are present in normal numbers in lymph node and spleen. These findings suggest a role for Fzd9 signaling in lymphoid development, particularly at points where B cells undergo self-renewal prior to further differentiation.
|
| pubmed:grant | |
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
AIM
|
| pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Fzd9 protein, mouse,
http://linkedlifedata.com/resource/pubmed/chemical/Immunoglobulin Light Chains,
http://linkedlifedata.com/resource/pubmed/chemical/Receptors, Neurotransmitter,
http://linkedlifedata.com/resource/pubmed/chemical/TCF Transcription Factors,
http://linkedlifedata.com/resource/pubmed/chemical/beta Catenin
|
| pubmed:status |
MEDLINE
|
| pubmed:month |
Mar
|
| pubmed:issn |
0006-4971
|
| pubmed:author | |
| pubmed:issnType |
Print
|
| pubmed:day |
15
|
| pubmed:volume |
105
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
2487-94
|
| pubmed:dateRevised |
2007-11-14
|
| pubmed:meshHeading |
pubmed-meshheading:15572594-Animals,
pubmed-meshheading:15572594-Atrophy,
pubmed-meshheading:15572594-B-Lymphocytes,
pubmed-meshheading:15572594-Bone Marrow Cells,
pubmed-meshheading:15572594-Bone Marrow Transplantation,
pubmed-meshheading:15572594-Cell Differentiation,
pubmed-meshheading:15572594-Chromosomes, Human, Pair 7,
pubmed-meshheading:15572594-Humans,
pubmed-meshheading:15572594-Immunoglobulin Light Chains,
pubmed-meshheading:15572594-Lymph Nodes,
pubmed-meshheading:15572594-Lymphatic Diseases,
pubmed-meshheading:15572594-Lymphopoiesis,
pubmed-meshheading:15572594-Mice,
pubmed-meshheading:15572594-Mice, Knockout,
pubmed-meshheading:15572594-Receptors, Neurotransmitter,
pubmed-meshheading:15572594-Signal Transduction,
pubmed-meshheading:15572594-Somatic Hypermutation, Immunoglobulin,
pubmed-meshheading:15572594-Spleen,
pubmed-meshheading:15572594-TCF Transcription Factors,
pubmed-meshheading:15572594-Thymus Gland,
pubmed-meshheading:15572594-Williams Syndrome,
pubmed-meshheading:15572594-beta Catenin
|
| pubmed:year |
2005
|
| pubmed:articleTitle |
Frizzled 9 knock-out mice have abnormal B-cell development.
|
| pubmed:affiliation |
Departments of Pathology and Genetics, Stanford University School of Medicine, Stanford, CA, USA. earanheim@wisc.edu
|
| pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't,
Research Support, N.I.H., Extramural
|